J Korean Pediatr Soc.
1994 Aug;37(8):1175-1181.
A Case of Anti-Neutrophil Cytoplasmic Antibodies (ABCA) Positive Wegener's Granulomatosis
Abstract
- Wegener's granulomatosis is a disease of unknown etiology that is characterized by the clinicopathologic complex of necrotixing granulomatous vasculitis of the upper and lower respiratory tract, glomerulonephritis, and variable degrees of small vessel vasculitis. Recently Antineutrophil Cytoplasmic Antibody (ANCA) has been reported to be a highly specific test for the diagnosis of Wegener's granulomatosis. We have experienced a patient of Wegener's granulomatosis in a 11 year old girl who was admitted with complaints f arthralgia, hematuria, convulsion and associated with otitis media and sinusitis. Serologic test of C-ANCA was positive and histologic findings of the kidney showed crescentic glomerulonephritis with sclerosis and surrounding infiltration of multinucleated giant cells. Patient was treated with pulse methylprednisolone without improvement. The clinical course progressed rapidly and expired due to the renal failure, gastrointestinal bleeding and status epilepticus. A brief review of literatures was made.
MeSH Terms
-
Antibodies, Antineutrophil Cytoplasmic*
Arthralgia
Child
Diagnosis
Female
Giant Cells
Glomerulonephritis
Hematuria
Hemorrhage
Humans
Kidney
Methylprednisolone
Otitis Media
Renal Insufficiency
Respiratory System
Sclerosis
Seizures
Serologic Tests
Sinusitis
Status Epilepticus
Vasculitis
Wegener Granulomatosis*
Antibodies, Antineutrophil Cytoplasmic
Methylprednisolone
